Characteristics of pulmonary arterial hypertension subgroups at the time of diagnosis, with a specific focus on demographics and treatment patterns
| CTD-PAH | IPAH | CHD-PAH | PoPH | PVOD | DPAH | CCB | HPAH | HIV-PAH | HHT-PAH | |
| Subjects, n | 80 (49) | 33 (20) | 22 (13) | 11 (7) | 5 (3) | 3 (2) | 3 (2) | 2 (1) | 2 (1) | 2 (1) |
| Sex: female n (%) | 72 (90) | 26 (79) | 13 (59) | 2 (18) | 2 (40) | 3 (100) | 3 (100) | 2 (100) | 2 (100) | 1 (50) |
| Age (years): mean±SD | 64±11 | 56±17 | 55±21 | 47±9 | 69±9 | 58±15 | 34±13 | 46±14 | 45±1 | 47±29 |
| WHO functional class: % I/II/III/IV | 0/22/60/18 | 3/24/55/18 | 0/50/50/0 | 0/36/64/0 | 0/20/40/40 | 0/33/33/33 | 0/67/33/0 | 0/0/100/0 | 0/50/50/0 | 0/50/50/0 |
| BNP (ng/L): mean±SD | 611±795 | 268±295 | 271±348 | 63±40 | 417±675 | 1330±1800 | 53±41 | 298±87 | --- | 187±127 |
| Risk stratification: % Low/intermediate/high risk | 8/61/31 | 27/45/28 | 27/55/18 | 9/91/0 | 20/60/20 | 33/33/33 | 33/67/0 | 0/50/50 | 50/50/0 | 0/100/0 |
| Right heart catheterisation (mean,±SD) | ||||||||||
| mPAP (mm Hg) | 43±11 | 50±12 | 51±25 | 51±17 | 38±14 | 43±25 | 38±1 | 49±0 | 44±6 | 46±0 |
| PAWP (mm Hg) | 11±4 | 8±3 | 13±5 | 12±7 | 12±10 | 7±0 | 7±2 | 8±0 | 12±0 | --- |
| CO (L/min) | 4±1 | 4±2 | 4±1 | 5±1 | 4±0 | 3±0 | 5±1 | 2±0 | 4±0 | 9±0 |
| PVR (WU) | 9±4 | 12±7 | 12±13 | 6±3 | 8±0 | 13±0 | 6±1 | 18±0 | 15±0 | --- |
| DLCO, % predicted | 35±14 | 69±20 | 73±21 | 50±6 | 43±13 | 19±0 | 88±0 | --- | --- | --- |
| PH therapy: n (%) | 79 (99) | 32 (97) | 14 (64) | 9 (82) | 5 (100) | 3 (100) | 1 (33) | 2 (100) | 2 (100) | 1 (50) |
| Monotherapy | 20 (25) | 4 (12) | 4 (29) | 4 (44) | 2 (40) | 1 (33) | 1 (100) | 0 | 1 (50) | 0 |
| PD5 inhib. | 10 (50) | 4 (100) | 2 (50) | 3 (75) | 2 (100) | 1 | 1 (100) | 0 | 0 | 0 |
| ERA | 10 (50) | 0 | 2 (50) | 1 (25) | 0 | 0 | 0 | 0 | 1 (100) | 0 |
| Double combination therapy | 45 (56) | 22 (67) | 9 (64) | 5 (56) | 2 (40) | 1 (33) | 0 | 2 (100) | 1 (50) | 1 (100) |
| PD5 inhib. and ERA | 44 (98) | 21 (95) | 9 (100) | 5 (100) | 2 (100) | 1 (100) | 0 | 2 (100) | 1 (100) | 1 (100) |
| sGCS+ERA | 0 | 1 (5) | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 |
| ERA+PGI2 | 1 (2) | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 |
| Triple combination therapy | 14 (18) | 6 (18) | 1 (7) | 0 | 1 (20) | 1 (33) | 0 | 0 | 0 | 0 |
| PD5 inhib.+ERA + neb PGI2 | 12 (86) | 4 (67) | 1 (100) | 0 | 1 (100) | 1 (100) | 0 | 0 | 0 | 0 |
| PD5 inhib.+ERA + oral PGI2 | 2 (14) | 2 (33) | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 |
Data were incomplete for the following characteristics and parameters at the time of diagnosis: mPAP was missing for 22 (13%) subjects, PAWP for 22 (13%), CO for 85 (52%), PVR for 95 (58%) and DLCO was missing in 87 (53%) cases. Of note: While mPAP was unavailable at diagnosis for 13% of PAH subjects, a subsequent RHC with evidence of a mPAP >25 mm Hg was available for all subjects, but not included in this analysis. Inadequate data were omitted and replaced by ‘---’.
BNP, B-type natriuretic peptide; CCB, long-term responsive to calcium channel blockers PAH; CHD-PAH, congenital heart disease associated PAH; CO, cardiac output; CTD-PAH, connective tissue disease associated pulmonary arterial hypertension; DLCO, diffusion capacity for carbon monoxide; DPAH, drug-associated PAH; ERA, endothelin receptor antagonist; HHT-PAH, hereditary haemorrhagic telangiectasia associated PAH; HIV-PAH, HIV associated PAH; HPAH, hereditary PAH; PD5 inhib, phosphodiesterase type-5 inhibitor; IPAH, idiopathic pulmonary arterial hypertension; mPAP, mean pulmonary artery pressure; neb, nebulised; PAWP, pulmonary artery wedge pressure; PGI2, prostacyclin; PH, pulmonary hypertension; PoPH, portopulmonary hypertension; PVOD, pulmonary veno occlusive disease; PVR, pulmonary vascular resistance; sGCS, soluble guanylate cyclase stimulator.